Subependymal giant cell astrocytoma is a benign brain tumor mostly associated with tuberous sclerosis complex. Recurrent subependymal giant cell astrocytoma in the absence of tuberous sclerosis. A 6-year-old spayed female Domestic Shorthair cat presented with a 1 to 2-month history of blindness and altered behavior. Shivaprasad NV, Satish S, Ravishankar S, Vimalambike MG. J Neurosci Rural Pract. Epub 2018 May 15. -, Ann Neurol. This website is intended for pathologists and laboratory personnel but not for patients. Histologically, subependymal nodules and subependymal giant cell tumours are essentially indistinguishable, and the distinction lies in the potential for growth and mass effect 5. They often result in obstructive hydrocephalus. However, it may be misinterpreted as other high-grade brain tumors due to … Original Article Subependymal giant cell astrocytoma (SEGA): Is it an astrocytoma? Ki-67 immunostaining in astrocytomas: Association with histopathological grade - A South Indian study. Nine patients (39.1%) had stigmata of tuberous sclerosis (6 at the time of diagnosis and 3 in the follow-up period). This lesion is larger than should be seen for other subependymal nodules in tuberous sclerosis. Sterman H, Furlan AB, Matushita H, Teixeira MJ. These 19 cases of SEGAs were collected over a period of 23 years (1979 to 2001), and accounted for 0.16% of intracranial tumors and 0.51% of all gliomas reported at our center. Management complicated by growth: Major ... Read more Management … MAHLON D. JOHNSON, JAMES B. ATKINSON, in Modern Surgical Pathology (Second Edition), 2009. 2016 Sep-Oct;35(5):295-301. doi: 10.5414/NP300936. Pharmacological treatment strategies for subependymal giant cell astrocytoma (SEGA). However there are several reported cases in which patients with a solitary SEGA had no other stigmata of TSC. USA.gov. Morphological, immunohistochemical and ultrastructural study. They are intraventricular and usually occur in the setting of tuberous sclerosis complex. The significance of the presence of T lymphocytes and mast cells is not clear. © Copyright PathologyOutlines.com, Inc. Click, Subependymal giant cell astrocytoma [title] (SEGA), Benign, slowly growing tumor typically arising in wall of lateral ventricles and composed of large ganglioid astrocytes, Usually associated with tuberous sclerosis, an autosomal dominant syndrome due to mutations in, Diagnostic criteria for tuberous sclerosis complex (TSC), modified from Roach et al. It usually arises from the medial portion of the lateral ventricle, grows into the lateral ventricle and may obstruct the foramen of Monro. Sharma MC(1), Ralte AM, Gaekwad S, Santosh V, Shankar SK, Sarkar C. Author information: (1)Department of Pathology, AIIMS, New Delhi, India. Front Neurol. Conspicuous proportion of neoplastic gemistocytes (> 20%) Neoplastic gemistocytes are angular shaped with abundant, glassy, eosinophilic cytoplasm and eccentric nuclei with distinct nucleoli They are intraventricular and usually occur in the setting of tuberous sclerosis complex. Subependymal giant cell astrocytomas are believed to arise from a subependymal nodule present in the ventricular wall in a patient with tuberous sclerosis, although this has yet to be categorica… The diagnosis is based on tissue, e.g. 1990;10(2):109-16 The tumor cells may be arranged in perivascular pseudorosettes. In view of its varied morphology, i.e. Subependymal giant cell astrocytoma shows large mostly polygonal cells with abundant cytoplasm and often vesicular eccentric nucleus with prominent nucleolus (a). Is mTOR Inhibitor Good Enough for Treatment All Tumors in TSC Patients? J Neurosurg Pediatr, 16(2):134-137, 15 May 2015 Cited by: 6 articles | PMID: 25978531 Subependymal Giant Cell Astrocytoma, Neurofilament immunohistochemical staining. Abstract Subependymal giant cell astrocytomas (SEGAs) are slowly growing tumours corresponding to WHO grade I. Epub 2020 Feb 26. In view of its varied morphology, i.e. 44 Symptomatic tumors occur in about 6% of patients with tuberous sclerosis complex, 44 … Six cases showed areas of necrosis and/or mitosis, but were not indicative of aggressive nature of this tumor. 2019 Jul 3;10:705. doi: 10.3389/fneur.2019.00705. It could be related to tumor immunology and may indicate a favorable prognosis. However, we cannot answer medical or research questions or give advice. Get the latest public health information from CDC: https://www.coronavirus.gov, Get the latest research information from NIH: https://www.nih.gov/coronavirus, Find NCBI SARS-CoV-2 literature, sequence, and clinical content: https://www.ncbi.nlm.nih.gov/sars-cov-2/. Clinical Characteristics of Subependymal Giant Cell Astrocytoma in Tuberous Sclerosis Complex. AFIP 1994, pp 102-105. Pathophysiology. Some (often circumscribed) astrocytic tumors are biologically different from diffuse astrocytomas An overview of CNS tumours is found in the CNS tumours article. Subependymal Giant Cell Astrocytoma SEGAs are uncommon WHO grade I intraventricular tumors that are most commonly associated with tuberous sclerosis complex (TSC). Microscopic examination showed varied histology consisting of sweeping bundles of spindle cells, gemistocyte and ganglion-like cells with interspersed inflammatory cell component. -, Acta Neuropathol. Epub 2020 Apr 27. Neurofibromatosis type-1 is a familial genetic syndrome associated with a predisposition to develop peripheral and central nervous system neoplasms. Subependymal giant cell astrocytoma is almost exclusively associated with tuberous sclerosis complex, which is an autosomal dominant disorder. We welcome suggestions or questions about using the website. A N A T O M I C A L P A T H O L O G Y Subependymal giant cell astrocytoma: a clinicopathological study of 23 cases with special emphasis on proliferative markers and expression of p53 and retinoblastoma gene proteins MEHAR C. SHARMA*, ANGELA M. RALTE*, RINA ARORA*, VANI SANTOSH{, S. K. SHANKAR{ AND CHITRA SARKAR* *Department of Pathology, All India Institute of … SUBEPENDYMAL GIANT CELL ASTROCYTOMA (WHO GRADE I) Clinical Presentation. Diagnosis. The average age at the time of surgery was 13.3 years. Individuals with very slow growing tumors where complete surgical removal by stereotactic surgery is possible may experience total remission. 1984;62(3):185-93 Epithelioid cells within Subependymal giant cell astrocytomas often react strongly with antibodies to neurofilaments. Pathogenesis of Tuberous Sclerosis Subependymal Giant Cell Astrocytomas: Biallelic Inactivation of TSC1 or TSC2 Leads to mTOR Activation Jennifer A. Chan, MD Division of Neuropathology, Department of Pathology, Brigham and Women's Hospital and Children's Hospital, Boston, Massachusetts Pathological examination revealed a subependymal giant-cell astrocytoma. The subependymal giant cell astrocytoma is a slow-growing neoplasm arising from a hamartoma of periventricular cells with neuronal and glial lineage differentiation, but its inclusion derives from its historical taxonomic relationship to astrocytomas. Methods: An institutional cohort of 105 brain tumors (51 dysembryoplastic neuroepithelial tumors (DNTs), 14 subependymal giant cell astrocytomas (SEGAs), 12 glioblastoma with neuronal marker expression (GBM-N), and 28 pleomorphic xanthoastrocytomas (PXAs)) from 100 patients were investigated for the presence of BRAF(V600E) by direct sequencing. Tumors of the central nervous system, Atlas of tumor pathology, 3rd series, fascicle #10. Subependymal giant cell astrocytoma -like astrocytomas have distinct clinicopathologic features. Pathology. The FLAIR image (left) demonstrates multiple subcortical hyperintense tubers. Subependymal giant cell astrocytomas (SEGAs) are relatively rare tumors but occur commonly in the setting of the familial syndrome of tuberous sclerosis complex (TSC). Beaumont TL, Godzik J, Dahiya S, Smyth MD. Would you like email updates of new search results? An astrocytoma is a neoplasm derived from an astrocyte.Diffuse astrocytomas are common glial tumours and grouped together with Oligodendroglioma in the current WHO brain tumor classficiation. Subependymal giant cell astrocytomas (SEGAs) are slowly growing tumours corresponding to WHO grade I. SUBEPENDYMAL GIANT CELL ASTROCYTOMA (WHO GRADE I) Clinical Presentation. Ocular giant cell astrocytoma, however, has been described in patients with and without the genetic mutation. Subependymal giant cell astrocytoma (SEGA): a case report and review of the literature. The FLAIR image (left) demonstrates multiple subcortical hyperintense tubers. CNS tumor - Gemistocytic astrocytoma IDH mutant. It is most commonly associated with tuberous sclerosis complex (TSC).Although it is a low-grade tumor, its location can potentially obstruct the ventricles and lead to hydrocephalus Subependymal giant cell Astrocytoma Zubair Ahmad, Fouzia Rauf, Najamul Sahar Azad, Aamir Ahsan Department of Pathology and Microbiology, Aga Khan University, Karachi. Epub 2008 Jun 17. Subependymal giant cell astrocytoma (SEGA, SGCA, or SGCT) is a low-grade astrocytic brain tumor (astrocytoma) that arises within the ventricles of the brain. eCollection 2019. 2013;29:335–9. They frequently contain cysts and calcification 8. Figure 1: This subependymal giant-cell astrocytoma (SEGA) is present in its typical location at the foramen of Monro. SEGA vs. SGTC: SEGA is most commonly known as the brain neoplasm associated with TSC (18, 24, 28).Historically, these tumors were considered to be astrocytomas. 8,9,19,20 Although relatively few cases have been reported, the most locally aggressive of these tumors contain calcium, display more than 50% … GFAP immunohistochemical staining preferentially reacts with fibrillary-appearing cellular elements within Subependymal giant cell astrocytoma. We studied 23 cases of SEGA, 19 from our own institute and 4 from NIMHANS, Bangalore. Childs Nerv Syst. Age ranged from 4 to 37 years (mean 13.2 years) with male predominance (M:F 2.2:1), and the duration of symptoms varied from 1 month to 96 months (mean 17.2 months). PubMed CrossRef Google Scholar 2004 Apr;36(2):139-44. Subependymal giant cell astrocytoma (SEGA) is a benign, slowly growing tumor typically occurring in the setting of tuberous sclerosis complex (TSC). We have previously reported on Last updated on Wednesday, April 8 2009 by gliageek. Childs Nerv Syst. In view of its varied morphology, i.e. Tahiri Elousrouti L, Lamchahab M, Bougtoub N, Elfatemi H, Chbani L, Harmouch T, Maaroufi M, Amarti Riffi A. J Med Case Rep. 2016 Feb 9;10:35. doi: 10.1186/s13256-016-0818-6. Last updated on Wednesday, April 8 2009 by gliageek. resemblance to astrocytic and ganglion cells, its histogenesis remains controversial. Buccoliero AM, Franchi A, Castiglione F, Gheri CF, Mussa F, Giordano F, Genitori L, Taddei GL. Subependymal giant cell astrocytoma is the most common CNS neoplasm associated with the tuberous sclerosis complex. Subependymal giant cell astrocytoma associated with tuberous sclerosis presenting with intratumoral bleeding. The majority of patients presented with visual disturbances (19/23, 82.6%) in the form of decreased vision (60.8%) and blindness (21.7%), generalized tonic clonic seizures (43.4%) and focal motor seizures (4.37%). Greenfield's Pathology of the central nervous system, 6th edition. Pilocytic astrocytoma; Subependymal giant cell astrocytoma; Subependymoma; Consist of slow growing astrocytomas, benign, and associated with long-term survival. The best way to distinguish it from a subependymal giant cell astrocytoma is the size. Subependymal nodule Subependymal giant cell astrocytoma Cardiac rhabdomyoma, single or multiple Lymphangiomatosis Renal angiomyolipoma Minor features. At necropsy, a 1-cm-diameter, firm … eCollection 2016. Buccoliero AM, Caporalini C, Giordano F, Mussa F, Scagnet M, Moscardi S, Baroni G, Genitori L, Taddei GL. 2009 Feb;29(1):25-30. doi: 10.1111/j.1440-1789.2008.00934.x. Subependymal giant cell astrocytoma--a clinicopathological study of 23 cases with special emphasis on histogenesis. 2020 Aug;21(11):1329-1336. doi: 10.1080/14656566.2020.1751124. Subependymal giant cell astrocytoma (SEGA): Is it an astrocytoma? Subependymal giant cell astrocytoma in the absence of tuberous sclerosis complex: case report. Patients included nine females and five males, with a mean age at diagnosis 28 years (range 4–60).  |  The inflammatory cell component on special staining turned out to be an admixture of mast cells and T lymphocytes. 1981 Feb;9(2):174-81 A search of the Mayo Clinic tissue registry yielded 73 giant cell-containing astrocytomas and intraventricular gliomas exclusive of ependymomas. Two patients died due to surgical complications, while the rest were alive and well in the follow-up period ranging from 3 to 264 months (mean 37.1 months). Case report and review of literature. 275 Vet Pathol 37:275–278 (2000) A Subependymal Giant Cell Astrocytoma in a Cat S. DUNIHO,F.Y.SCHULMAN,A.MORRISON,H.MENA, AND A. KOESTNER Abstract. Neuropathology. It usually arises from the medial portion of the lateral ventricle, grows into the lateral ventricle and may obstruct the foramen of Monro. This site needs JavaScript to work properly. These tumors are multilobulated well-circumscribed tumors arising from the wall of the lateral ventricles near the foramen of Monro. However there are several reported cases in which patients with a solitary SEGA had no other stigmata of TSC. Pathology. (, Nontraumatic ungual or periungual fibroma, Multiple randomly distributed pits in dental enamel, Tuberous sclerosis also associated with pulmonary and uterine lymphangiomyomatosis, renal angiomyolipoma, cardiac rhabdomyoma, SEGA present in 6% of tuberous sclerosis patients, 20 year old woman with solitary subependymal giant cell astrocytoma and mutation of, Composed mainly of large polygonal to elongate cells resembling astrocytes or ganglion cells with abundant, finely granular eosinophilic cytoplasm, bright pink cellular processes, large round / oval nuclei, prominent nucleoli, Perivascular pseudorosette formation is common, Infiltration of mast cells and lymphocytes is common, Presence of mitoses, vascular proliferation or necrosis does NOT indicate anaplastic progression. We studied 23 cases of SEGA, 19 from our own institute and 4 from NIMHANS, Bangalore. They frequently contain cysts and calcification 8. a biopsy. 625-627. 44 Symptomatic tumors occur in about 6% of patients with tuberous sclerosis complex, 44 … -, Pathology. COVID-19 is an emerging, rapidly evolving situation. An astrocytoma is a neoplasm derived from an astrocyte.Diffuse astrocytomas are common glial tumours and grouped together with Oligodendroglioma in the current WHO brain tumor classficiation.  |  The clinical history, neuroimaging features, treatments, and outcome of 4 patients with histologically verified subependymal giant cell astrocytomas (SEGA) were retrospectively reviewed. Subependymal giant cell astrocytoma (SEGA) is a benign, slowly growing tumor typically occurring in the setting of tuberous sclerosis complex (TSC). Fohlen M, Ferrand-Sorbets S, Delalande O, Dorfmüller G. Childs Nerv Syst. Subependymal giant cell astrocytoma (SEGA) is a benign, slowly growing tumor typically occurring in the setting of tuberous sclerosis complex (TSC) with long-term survival. Visual survey of surgical pathology with 10923 high-quality images of benign and malignant neoplasms & related entities. resemblance to astrocytic and ganglion cells, its histogenesis remains controversial. These tumours are small, no more than two centimeters across, coming from the ependyma. It usually arises from the medial portion of the lateral ventricle, grows into the lateral ventricle and may obstruct the foramen of … Subependymal giant cell astrocytoma (SEGA) is a benign, slowly growing tumor typically occurring in the setting of tuberous sclerosis complex (TSC) with long-term survival. A 13-year-old boy presented with an obstructive left lateral intraventricular mass. The subependymal giant cell astrocytoma is a slow-growing neoplasm arising from a hamartoma of periventricular cells with neuronal and glial lineage differentiation, but its inclusion derives from its historical taxonomic relationship to astrocytomas. Follow Dr. Pernick's blog by clicking, 30100 Telegraph Road, Suite 408, Bingham Farms, Michigan 48025 (USA). These tumours are multilobulated well-circumscribed tumours arising from the wall of the lateral ventricles near the foramen of Monro. Subependymal giant cell astrocytoma: a lesion with activated mTOR pathway and constant expression of glutamine synthetase. Sharma M(1), Ralte A, Arora R, Santosh V, Shankar SK, Sarkar C.  |  Ann Pathol. Subependymal giant cell astrocytoma is the most common CNS neoplasm associated with the tuberous sclerosis complex. This lesion is larger than should be seen for other subependymal nodules in tuberous sclerosis. Initial Management at Presentation Early surgical removal: Early tumor resection is advocated in asymptomatic children having SEGA with TSC, primarily when there is evidence of growth on successive MRIs (2, 7, 10, 18, 24, 26, 27). Subependymal giant cell astrocytoma: a clinicopathological study of 23 cases with special emphasis on proliferative markers and expression of p53 and retinoblastoma gene proteins. Subependymal giant cell astrocytoma associated with tuberous sclerosis presenting with intratumoral bleeding. How does cancer arise based on complexity theory? Jansen AC, Belousova E, Benedik MP, Carter T, Cottin V, Curatolo P, Dahlin M, D'Amato L, d'Augères GB, de Vries PJ, Ferreira JC, Feucht M, Fladrowski C, Hertzberg C, Jozwiak S, Lawson JA, Macaya A, Marques R, Nabbout R, O'Callaghan F, Qin J, Sander V, Sauter M, Shah S, Takahashi Y, Touraine R, Youroukos S, Zonnenberg B, Kingswood JC. 2016 Jul 21;7(12):1621-1631. doi: 10.7150/jca.14747. Identification of TSC1 or TSC2 mutation limited to the tumor in three cases of solitary subependymal giant cell astrocytoma using next-generation sequencing technology. TSC is an autosomal dominantly inherited neurocutaneous syndrome that affects any organ system of the body. Original Article Subependymal giant cell astrocytoma (SEGA): Is it an astrocytoma? Expert Opin Pharmacother. Two patients experienced recurrences, one two years and another 22 years after surgery. A 6-year-old spayed female Domestic Shorthair cat presented with a 1 to 2-month history of Multiple randomly distributed pits in dental enamel Hamartomatous rectal polyps Bone cysts Cerebral white matter migration lines Gingival fibromas Nonrenal hamartomas Retinal achromic patch Subependymal giant cell astrocytoma is a benign brain tumor mostly associated with tuberous sclerosis complex. Please enable it to take advantage of the complete set of features! A search of the Mayo Clinic tissue registry yielded 73 giant cell-containing astrocytomas and intraventricular gliomas exclusive of ependymomas. Surgery for subependymal giant cell astrocytomas in children with tuberous sclerosis complex. The best way to distinguish it from a subependymal giant cell astrocytoma is the size. Immunoreactivity for GFAP, NF, S-100, NSE and synaptophysin indicates that this is a hybrid tumor with glial and neuronal differentiation. 1981;53(2):113-7 Graham, DI, Lantos PL. Twenty-two cases of subependymal giant cell astrocytoma (SGCA), five of which associated with tuberous sclerosis, were reviewed by conventional neurohistological stains and by peroxidase-antiperoxidase (PAP) immunohistochemistry for glial fibrillary acidic (GFA) protein, the 68 Kd neurofilament subunit (68 Kd-NF), and neuron-specific enolase (NSE). The diagnosis is based on tissue, e.g. Subependymal giant cell astrocytomas (SEGAs) are slowly growing tumours corresponding to WHO grade I. Mcgraw Hill, 1994. Pathology. 2020 May;36(5):961-965. doi: 10.1007/s00381-020-04551-4. We stud … Some (often circumscribed) astrocytic tumors are biologically different from diffuse astrocytomas An overview of CNS tumours is found in the CNS tumours article. Habib SL, Al-Obaidi NY, Nowacki M, Pietkun K, Zegarska B, Kloskowski T, Zegarski W, Drewa T, Medina EA, Zhao Z, Liang S. J Cancer. HHS Week 731: Case 3; Week 731: Case 2; Week 731: Case 1; ... We’d like to send you periodic updates regarding Pathology educational materials released by our department. Comments: Subependymal giant cell astrocytoma is usually seen in children or young adults in the setting of tuberous sclerosis (5% to 15% of cases).It is a well-demarcated, non-infiltrating, solid mass that is composed of epithelioid, gemistocyte-like, and spindled cells.Several gemistocyte-like cells can be seen in this field. Subependymal giant cell astrocytoma (SEGA) is a benign, slowly growing tumor typically occurring in the setting of tuberous sclerosis complex (TSC) with long-term survival. They often result in obstructive hydrocephalus. Webpathology.com: A Collection of Surgical Pathology Images Subependymal Giant Cell Astrocytoma Diagnosis. a biopsy. None of the tumors was immunopositive for HMB-45. -, Acta Neuropathol. Subependymal giant cell astrocytoma (SEGA) is a benign, slowly growing tumor typically occurring in the setting of tuberous sclerosis complex (TSC). They are intraventricular and usually occur in the setting of tuber- The cells that appear astrocytic, usually resemble gemistocytes; large … 2013;29:335–9. Search by Diagnosis: "Subependymal giant cell astrocytoma" Show Diagnoses Week 40: Case 2 Diagnosis: Subependymal giant cell astrocytoma. Subependymal giant cell astrocytomas are considered WHO grade I lesions in the current (2016) WHO classification of CNS tumours 8. Summary. The authors describe five cases of subependymal giant-cell astrocytoma in children in which many clinical, histological, immunohistochemical, and ultrastructural features typical of … resemblance to astrocytic and ganglion cells, its histogenesis remains controversial. NIH Tumors are pathological - ly classified as grade I … In 20 of the 23, histological or clinical evidence showed the tumor to be a subependymal giant cell astrocytoma. Subependymal giant cell astrocytomas (SEGAs) occur in approximately 6% of patients with tuberous sclerosis (TS) and are often considered to be a forme fruste or partial expression of this disorder. NLM Subependymal giant cell astrocytomas are considered WHO grade I lesions in the current (2016) WHO classification of CNS tumors 8. 1. Subependymal Giant Cell Astrocytoma, GFAP immunohistochemical staining x 400. National Center for Biotechnology Information, Unable to load your collection due to an error, Unable to load your delegates due to an error. Lateral ventricular involvement was the most common site (91.3%), followed by the third ventricle (8.6%). Clin Neuropathol. Subependymal giant cell astrocytoma (SEGA) is a benign slowly growing tumor, which typically arises at the caudotha-lamic groove adjacent to the foramen of Monro and is com-posed of large ganglioid astrocytes [1]. Xue Song, Qayyim Said, Oth Tran, Darcy A. Krueger, John Bissler, Everolimus compliance and persistence among tuberous sclerosis complex patients with renal angiomyolipoma or subependymal giant cell astrocytoma, Current Medical Research and Opinion, 10.1080/03007995.2018.1558883, (1-8), (2018). MAHLON D. JOHNSON, JAMES B. ATKINSON, in Modern Surgical Pathology (Second Edition), 2009. 2004 Apr;36(2):139-44. doi: 10.1080/0031302410001671975. Growth can lead to sudden death due to acute hydrocephalus and intraventricular bleeding (33). Fohlen M, Harzallah I, Polivka M, Giuliano F, Pons L, Streichenberger N, Dorfmüller G, Touraine R. Childs Nerv Syst. Case report and review of literature. -. Subependymal giant cell astrocytomas (SEGAs) are relatively rare tumors but occur commonly in the setting of the familial syndrome of tuberous sclerosis complex (TSC). Recent Cases. These tumours are small, no more than two centimeters across, coming from the ependyma. vajdler jr.1, Ladislav Deák2, Boris Rychl˘3, Peter Talarãík3, Lucia Fröhlichová1 1Department of pathology, L. Pasteur’s University Hospital, Ko‰ice, Slovakia 2016 Oct-Dec;7(4):510-514. doi: 10.4103/0976-3147.188626. Subependymal giant cell astrocytomas (SEGAs) are relatively rare tumors but occur commonly in the setting of the familial syndrome of tuberous sclerosis complex (TSC). However there are several reported cases in which patients with a solitary SEGA had no other stigmata of TSC. Subependymal giant cell astrocytomas are believed to arise from a subependymal nodule present in the ventricular wall in a patient with tuberous sclerosis, although this has yet to be categorica… Ultrastructural examination confirmed previously reported features of … Subependymal giant cell astrocytoma: a clinicopathological study of 23 cases with special emphasis on proliferative markers and expression of p53 and retinoblastoma gene proteins. Sterman H, Furlan AB, Matushita H, Teixeira MJ. Further clinical investigation confirmed the diagnosis of tuberous sclerosis. 2018 Aug;34(8):1511-1519. doi: 10.1007/s00381-018-3826-6. The prevalence rate of … Sharma M, Ralte A, Arora R, Santosh V, Shankar SK, Sarkar C. Pathology. Figure 1: This subependymal giant-cell astrocytoma (SEGA) is present in its typical location at the foramen of Monro. Clipboard, Search History, and several other advanced features are temporarily unavailable. The authors describe five cases of subependymal giant-cell astrocytoma in children in which many clinical, histological, immunohistochemical, and ultrastructural … 1991 Jul;23(3):185-8 A subset of subependymal giant cell astrocytoma-like astrocytomas are alternative lenghtening of telomere-positive and occur in the absence of ATRX alterations, thereby suggesting mutations in other DNA repair/maintenance genes may also facilitate alternative lenghtening of telomeres. Halmagyi, G Micheal et al. Subependymal giant cell astrocytoma-like astrocytoma: a neoplasm with a distinct phenotype and frequent neurofibromatosis type-1-association. PubMed CrossRef Google Scholar In 20 of the 23, histological or clinical evidence showed the tumor to be a subependymal giant cell astrocytoma. Subependymal giant cell astrocytoma (SEGA) is a clinically benign tumor that is usually associated with tuberous sclerosis complex (TSC) .